February 1990
Volume 31, Issue 2
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Articles  |   February 1990
A form of congenital stationary night blindness with apparent defect of rod phototransduction.
Author Affiliations
  • N S Peachey
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
  • G A Fishman
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
  • P E Kilbride
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
  • K R Alexander
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
  • K M Keehan
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
  • D J Derlacki
    Department of Ophthalmology, University of Illinois, Chicago College of Medicine 60612.
Investigative Ophthalmology & Visual Science February 1990, Vol.31, 237-246. doi:
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      N S Peachey, G A Fishman, P E Kilbride, K R Alexander, K M Keehan, D J Derlacki; A form of congenital stationary night blindness with apparent defect of rod phototransduction.. Invest. Ophthalmol. Vis. Sci. 1990;31(2):237-246.

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Abstract

We report findings obtained from an individual with an unusual form of congenital stationary night blindness (CSNB). Although the rhodopsin density difference of this subject was normal, there was no evidence of rod-mediated visual function. Dark-adapted thresholds were cone-mediated, and dark-adapted electroretinograms (ERGs) represented activity of the cone system exclusively. ERG a- and b-waves obtained under light-adapted conditions were normal. The absence of a rod a-wave but the presence of normal rhodopsin density, in combination with normal cone function, indicates that this form of CSNB likely involves a defect of phototransduction that is limited to the rods. In addition, light-adapted b-wave responses to high luminance flashes were larger than dark-adapted responses, whereas a-wave amplitudes were reduced by light adaptation. These ERG results address proposed mechanisms by which light adaptation might enhance cone system responses.

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